Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2025 Jun 21:21:100602.
doi: 10.1016/j.ijcchd.2025.100602. eCollection 2025 Sep.

Trends in mortality from pulmonary hypertension amongst population with congenital heart disease in the United States from 1999 to 2020: A CDC WONDER analysis

Affiliations

Trends in mortality from pulmonary hypertension amongst population with congenital heart disease in the United States from 1999 to 2020: A CDC WONDER analysis

Allahdad Khan et al. Int J Cardiol Congenit Heart Dis. .

Abstract

Background: Congenital heart disease (CHD) is an umbrella term describing a variety of structural cardiac malformations at birth. CHD affects approximately 1 % of live births, generating a large adult population with these abnormalities. Pulmonary hypertension (PH) in patients with adult CHD is heterogeneous, based on the type of defect and associated conditions, but is a known cause of adverse outcome.

Method: We retrieved death certificate data from the CDC-WONDER database using ICD codes (I27.0, I27.2, I27.8, and I27.9., and Q20-26). Crude mortality rates (CMRs) and age-adjusted mortality rates (AAMRs) per 100,000 persons were calculated. Temporal trends were examined using the annual percent change (APC) and average annual percent change (AAPC) determined by Joinpoint regression.

Result: From 1999 to 2020, an overall declining pattern was observed in the mortality rate. Men and women with congenital heart disease and pulmonary hypertension in the U.S. experienced a statistically significant decline in mortality rates. In terms of races, among White individuals, the decline was the most pronounced. All four U.S. census regions experienced statistically significant declines in mortality due to pulmonary hypertension among individuals with congenital heart disease. The rate of decline was steeper in rural areas compared to urban ones.

Conclusion: This study highlights that previously implemented targeted interventions significantly contributed to the reduction of mortality amongst patiemnts with congenital heart disease and pulmonary hypertension in the U.S. Still improvements are required in certain areas, including female gender, Hispanic or Latino, and the Northeast and West regions.

Keywords: CDC WONDER; Congenital heart disease; Mortality; Pulmonary hypertension.

PubMed Disclaimer

Conflict of interest statement

The authors declare the following financial interests/personal relationships which may be considered as potential competing interests:Raheel Ahmed reports was provided by Imperial College London National Heart and Lung Institute. If there are other authors, they declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.

Figures

Image 1
Graphical abstract
Fig. 1
Fig. 1
Age-adjusted mortality rates (AAMRs) per 100,000 individuals stratified by sex/gender in the United States, 1999 to 2020.
Fig. 2
Fig. 2
Age-adjusted mortality rates (AAMRs) per 100,000 individuals stratified by race/ethnicity in the United States, 1999 to 2020.
Fig. 3
Fig. 3
Age-adjusted mortality rates (AAMRs) per 100,000 individuals stratified by census region in the United States, 1999 to 2020.
Fig. 4
Fig. 4
Age-adjusted mortality rates (AAMRs) per 100,000 individuals stratified by urbanization in the United States, 1999 to 2020.
Fig. 5
Fig. 5
Age-adjusted mortality rates (AAMRs) per 100,000 individuals stratified by state in the United States, 1999 to 2020.

References

    1. Meng X., Song M., Zhang K., Lu W., Li Y., Zhang C., Zhang Y. Congenital heart disease: types, pathophysiology, diagnosis, and treatment options. MedComm. 2020;5(7) doi: 10.1002/mco2.631. 2024 Jul 5. - DOI - PMC - PubMed
    1. Centers for Disease Control and Prevention (CDC) Congenital heart defects (CHDs) 2025. https://www.cdc.gov/heart-defects/about/index.html#:∼:text=What%20it%20i... [Internet]. Available from:
    1. Oldroyd S.H., Manek G., Bhardwaj A. StatPearls. StatPearls Publishing; Treasure Island (FL): 2025 Jan. Pulmonary hypertension.https://www.ncbi.nlm.nih.gov/books/NBK482463/ [Updated 2024 May 1]
    1. Suresh Kumar Vasupradha, Sable, Craig Nakiwala, Dorothy Kassebaum, Nicholas Abstract 4142897: the burden of adult congenital heart disease in the United States. Circulation. A4142897-A4142897. 150. Suppl_1. 2024 doi: 10.1161/circ.150.suppl_1.4142897. https://www.ahajournals.org/doi/abs/10.1161/circ.150.suppl_1.4142897 - DOI - DOI
    1. Roos-Hesselink Jolien W., Pelosi Chiara, Brida Margarita, De Backer Julie, Ernst Sabine, Werner Budts, Baumgartner Helmut, Oechslin Erwin, Tobler Daniel, Kovacs Adrienne H., Di Salvo Giovanni, Kluin Jolanda, Gatzoulis Michael A., Diller Gerhard P. Surveillance of adults with congenital heart disease: current guidelines and actual clinical practice. Int J Cardiol. 2024;407 doi: 10.1016/j.ijcard.2024.132022. ISSN 0167-5273. - DOI - PubMed